Aim: To determine the frequency and clinical features of corpus callosum hypoagenesis, dysgenesis or hypoplasia in conjunction with extraparenchymal interhemispheric cyst. Methods: A retrospective study of clinical fi...Aim: To determine the frequency and clinical features of corpus callosum hypoagenesis, dysgenesis or hypoplasia in conjunction with extraparenchymal interhemispheric cyst. Methods: A retrospective study of clinical files and imaging records of all 2500 children referred to the University Department of Paediatrics, Catania, Italy, who underwent neuroimaging by ultrasound, computerized tomography or magnetic resonance imaging in 1992-2003. Five children (all girls, aged 2-18 y) were found to have abnormalities of the corpus callosum associated with interhemispheric cyst. Results: Three cases were type 2b, one type 2c and one mixed type 2b and 2c according to the classification proposed by Barkovich et al. The onset of clinical symptoms was very early, with severe neurological involvement, seizures that were difficult to treat and profound psychomotor retardation. Conclusions: In callosal anomalies with cysts, a prevalence in females is not confined to type 2b cysts, the spectrum of abnormalities of the corpus callosum is more varied than previously recorded, and the natural history and outcome of the condition are poor with profound developmental delay and drug-resistant seizures. The finding that all the cases were of type 2 suggests that the overall phenotype is more widespread than previously thought andmay present in association with other complex syndromes.展开更多
We carried out an epidemiological survey to determine prevalence and incidence of multiple sclerosis in the little town of Linguaglossa in the Province of Catania. We calculated prevalence rate as point prevalence at ...We carried out an epidemiological survey to determine prevalence and incidence of multiple sclerosis in the little town of Linguaglossa in the Province of Catania. We calculated prevalence rate as point prevalence at 1 January 2001 and incidence during 1991-2000. We studied the frequency of multiple sclerosis in the community of Linguaglossa in a population of 5,422 inhabitants in the 2001 census. The primary sources for the case ascertainment were the general practitioners of Linguaglossa, the local Italian Multiple Sclerosis Association and the neurological departments, Multiple Sclerosis Centers and private neurologists of the province of Catania. We considered as prevalent and incident cases all patients who satisfied the Poser’s diagnostic criteria. We detected 11 patients with multiple sclerosis who had had the onset of disease on prevalent day (P.D.). The onset-adjusted prevalence rate was 203/100,000 (95%CI 107-352). Prevalence was higher in women (247/100,000) than in men (154/100,000). From 1991 to 2000, 10 subjects with MS had clinical onset of disease. The mean annual incidence risk was 18.2/100,000 (C. I. 95 %5.9-42.5). Conversely in the same population prevalence on 1 January 1991 was 37/100,000 while the onset adjusted annual incidence risk during the previous decade (1981-1991) was 3.6/100,000. Prevalence and incidence rates of MS during the last decade in the little town of Linguaglossa are higher than those found in the same area during the previous ten years and also than those reported in other Sicilian and Italian surveys suggesting a possible cluster of MS.展开更多
文摘Aim: To determine the frequency and clinical features of corpus callosum hypoagenesis, dysgenesis or hypoplasia in conjunction with extraparenchymal interhemispheric cyst. Methods: A retrospective study of clinical files and imaging records of all 2500 children referred to the University Department of Paediatrics, Catania, Italy, who underwent neuroimaging by ultrasound, computerized tomography or magnetic resonance imaging in 1992-2003. Five children (all girls, aged 2-18 y) were found to have abnormalities of the corpus callosum associated with interhemispheric cyst. Results: Three cases were type 2b, one type 2c and one mixed type 2b and 2c according to the classification proposed by Barkovich et al. The onset of clinical symptoms was very early, with severe neurological involvement, seizures that were difficult to treat and profound psychomotor retardation. Conclusions: In callosal anomalies with cysts, a prevalence in females is not confined to type 2b cysts, the spectrum of abnormalities of the corpus callosum is more varied than previously recorded, and the natural history and outcome of the condition are poor with profound developmental delay and drug-resistant seizures. The finding that all the cases were of type 2 suggests that the overall phenotype is more widespread than previously thought andmay present in association with other complex syndromes.
文摘We carried out an epidemiological survey to determine prevalence and incidence of multiple sclerosis in the little town of Linguaglossa in the Province of Catania. We calculated prevalence rate as point prevalence at 1 January 2001 and incidence during 1991-2000. We studied the frequency of multiple sclerosis in the community of Linguaglossa in a population of 5,422 inhabitants in the 2001 census. The primary sources for the case ascertainment were the general practitioners of Linguaglossa, the local Italian Multiple Sclerosis Association and the neurological departments, Multiple Sclerosis Centers and private neurologists of the province of Catania. We considered as prevalent and incident cases all patients who satisfied the Poser’s diagnostic criteria. We detected 11 patients with multiple sclerosis who had had the onset of disease on prevalent day (P.D.). The onset-adjusted prevalence rate was 203/100,000 (95%CI 107-352). Prevalence was higher in women (247/100,000) than in men (154/100,000). From 1991 to 2000, 10 subjects with MS had clinical onset of disease. The mean annual incidence risk was 18.2/100,000 (C. I. 95 %5.9-42.5). Conversely in the same population prevalence on 1 January 1991 was 37/100,000 while the onset adjusted annual incidence risk during the previous decade (1981-1991) was 3.6/100,000. Prevalence and incidence rates of MS during the last decade in the little town of Linguaglossa are higher than those found in the same area during the previous ten years and also than those reported in other Sicilian and Italian surveys suggesting a possible cluster of MS.
